Desmoplastic fibroma of bone is an extremely rare tumor that was first described by Jaffe in 1958. It histologically resembles the desmoid tumor of soft tissue. It is known as locally aggressive tumor but we experienced definitely benign and resembling simple bone cyst radiographically. We report a case of desmoplastic fibroma of bone and it should be included in the differential diagnosis list of any lytic bone lesion. The radiograph, MR imaging features, radiological and pathological differential diagnosis of the case are described, and literatures are reviewed.
Bone Cysts ; Diagnosis, Differential ; Female ; Fibroma, Desmoplastic ; Fibromatosis, Aggressive ; Humans

OBJECTIVE: To evaluate the clinic manifestation, pathologic behavior, therapy and prognosis of rare aggressive fibromatosis in the head and neck. METHOD: Two cases of aggressive fibromatosis were analyzed and relevant literatures were reviewed. RESULT: Aggressive fibromatosis was characterized as infiltrative, locally aggressive and tended to recur after surgical resection. Pathology showed fibroblastic monoclonal proliferation. Fibromatosis was composed of well-differentiated fibroblasts and myofibroblasts, lacking cytological features of malignancy and scanty or absent mitotic activity. Complete surgical excision of aggressive fibromatosis was considered to be the only effective method of cure by most authorities. Chemotherapy and radiotherapy can be used together with surgery in recurrence or unsatisfactory surgical margin. In our study, one patient recurred after the first operation, and after another operation, the patient did not recur after 6 months follow up, and the other one did not recur after 6 months follow up. CONCLUSION The diagnosis of aggressive fibromatosis depended on pathological examination. Radical removal was an important way to reduce recurrence rate. Radiation therapy and chemotherapy can be used as adjuvant therapy in patients with recurrent or unresectable or inoperable disease.
Adolescent ; Female ; Fibromatosis, Aggressive ; diagnosis ; surgery ; Head and Neck Neoplasms ; diagnosis ; surgery ; Humans ; Middle Aged

Intra-abdominal desmoid tumor (IADT) and gastrointestinal stromal tumor (GIST) are both mesenchymal tumors mostly found in gastrointestinal tracts and easily misdiagnosed, which would directly damage the survival prognosis and quality of life of patients. With the advent of the era of precision medicine, the understanding of the above two diseases is more in-depth, and the requirements for accurate diagnosis and individualized precision treatment are more stringent. Moreover, there seems to be some internal relationship between IADT and GIST, and the lack of systematic research and discussion makes clinical decision-making and patient management easy to fall into traps and misunderstandings. Therefore, this paper reviews the clinical characteristics, pathogenesis and treatments of the two, and explore their differences and internal relations, so as to provide research and practical reference for promoting more precise and individualized diagnosis and treatment regimens.
Clinical Decision-Making ; Fibromatosis, Aggressive/diagnosis* ; Gastrointestinal Stromal Tumors/diagnosis* ; Humans ; Prognosis ; Quality of Life

Mesenteric fibromatosis is a rare benign fibrous tumor that can occur from bowel mesentery of the retroperitoneum. It can infiltrate the surrounding structures and tends to recur locally even after resection but does not have metastatic capability. Mesenteric fibromatosis represents 8% of all intra-abdominal desmoid neoplasm. We experienced a case of mesenteric fibromatosis in a 50-year-old woman with a painless abdominal mass. An exploratory laparotomy was performed, and two large, small bowel mesenteric masses were found which were invading the transverse colon. The segment of the jejunum and transverse colon including the masses were resected widely and the pathologic report confirmed the diagnosis of fibromatosis. We reviewed the features of the mesenteric fibromatosis, that is, clinical, imaging, pathological, immunohistological features, and differential diagnosis and treatment of mesenteric fibromatosis.
Colon, Transverse ; Diagnosis, Differential ; Female ; Fibroma ; Fibromatosis, Aggressive ; Humans ; Jejunum ; Laparotomy ; Mesentery ; Middle Aged

A mass excision surrounding the bronchial stump was performed to exclude malignancy in a 42-year-old man who had undergone a right lower lobectomy for lung cancer. The mass was identified as a cicatricial fibroma. Cicatricial fibromatosis, which is desmoid fibromatosis that arises in a surgical scar, is a well-known clinical condition. It consists of histologically benign neoplasms. Their occurrence after thoracic surgery is extremely rare. Biopsy or excision of suspicious lesions is very important for diagnosis. R0 resection remains the principal outcome for intra-thoracic desmoid fibromatosis. We report that a cicatricial fibromatosis in the subcarinal space was removed after suspicion of local recurrence at the bronchial stump follwing lobectomy for lung cancer.
Adult ; Biopsy ; Cicatrix ; Diagnosis* ; Fibroma* ; Fibromatosis, Aggressive ; Humans ; Lung Neoplasms* ; Lung* ; Recurrence* ; Thoracic Surgery

Desmoid tumors are rare soft tissue tumors arising from the fascia or from musculoaponeurotic structures. They are commonly seen in the extremities, but are rarely found in the thorax. Thoracic desmoid tumors commonly arise from the chest wall and rarely in the thoracic cavity. Imaging diagnosis of an intrathoracic desmoid tumor is difficult because there are no specific imaging findings for a desmoid tumor that can be differentiated from the various tumors of the chest wall, including a solitary fibrous tumor of the pleura. All desmoid tumor cells show negative immunohistochemical staining for CD34 in pathological specimen, a feature that makes it possible to differentiate a desmoid tumor from a solitary fibrous tumor of the pleura. Desmoid tumors are locally aggressive and the rate of local recurrence is very high. Consequently, wide radical resection is required and a preoperative accurate diagnosis of desmoid tumors is warranted. We describe the radiological findings of various imaging studies for an intrathoracic desmoid tumor. Our findings should facilitate a proper diagnosis of desmoid tumors.
Diagnosis ; Extremities ; Fascia ; Fibromatosis, Aggressive* ; Recurrence ; Solitary Fibrous Tumor, Pleural ; Thoracic Cavity ; Thoracic Wall ; Thorax

PURPOSE: The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related DTs. MATERIALS AND METHODS: We reviewed the medical records and database of all patients with FAP who were treated between January 1993 and December 2011. RESULTS: Of 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominal DTs and one had intra- and extra-abdominal DTs. We divided the patients into two groups according to type of resection; the R0 or R1 resection group, referred to as the curative resection group (eight patients), and the R2 resection/palliative operation/medical treatment group, referred to as the palliative resection group (10 patients). Two patients in the curative resection group and two patients in the palliative group had progressed to tumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up. CONCLUSION: A multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatory drugs, anti-estrogens, cytotoxic agents, and surgery. However, the role of surgery in resectable and complicated tumors may be limited. DT unrelated to surgical trauma has a relatively poor prognosis.
Adenomatous Polyposis Coli* ; Cytotoxins ; Diagnosis ; Fibromatosis, Aggressive* ; Follow-Up Studies ; Humans ; Medical Records ; Prognosis ; Risk Factors

Pyourachus is an infection of urachal cyst which is one of congenital urachal anormalies. It should bedifferentiated from urachal tumor in adult, and abdominal desmoplastic round cell tumor, non-Hodgkin's lymphoma,and intra-abdominal desmoid tumor in children. The CT findings of pyourachus are characteristic to provide anaccurate preoperative diagnosis. The authors encountered a case of pyourachus in a 58-year-old male and report thecharacteristic CT findings. A large hypodense spherical mass with irregular thickened wall was present in themidline of the lower abdomen under the rectus abdominalis muscle extending from the dome of the urinary bladder tothe level of the umbilicus.
Abdomen ; Adult ; Child ; Diagnosis ; Fibromatosis, Aggressive ; Humans ; Male ; Middle Aged ; Umbilicus ; Urachal Cyst ; Urachus ; Urinary Bladder

Nine cases of mesenteric desmoid-type fibromatosis were diagnosed and treated in Taizhou Hospital, Wenzhou Medical University between January 2010 and May 2022, including 2 females and 7 males, aged 16 to 59 years. The lesions were in the mesentery of small intestine with 7 cases, ileocecal junction with 1 cases and transverse colon with 1 case. The tumors had an unclear boundary and no envelope, the section was solid, gray and tough. The mean maximum diameter was (10.7±8.5) cm (range 3.5-33.0 cm). Microscopically, fusiform fibroblasts and myofibroblasts were parallel, bunched or staggered, buried in a large amount of extracellular collagen. The cell morphology was relatively consistent, without obvious atypia, and mitosis was rare. Immunohistochemistry showed that the tumor cells were positive for vimentin (9/9), β-catenin (9/9), while smooth muscle actin (5/9) stains were focally positive. Ki-67 proliferation index was 1%-10%. Cytokeratin Pan, S-100, STAT6, CD117, DOG1, CD34, desmin and anaplastic lymphoma kinase stains were negative. Genetic analysis showed that there were 7 cases of c.121G>A(p.Thr41Ala) mutation of CTNNB1 gene, 1 case of c.121G>A(p.Thr41Ala) and 1 case of c.134C>T(p.Ser45Phe) double mutation, and 1 case of wild type. Tumors were surgically resected in all 9 cases. Eight cases had no recurrence or metastasis, 1 case had recurrence 6 months later, and no recurrence or metastasis after additional surgical resection.
Male ; Female ; Humans ; Fibromatosis, Aggressive/diagnosis* ; Immunohistochemistry ; Fibroblasts/metabolism* ; Mesentery/pathology* ; beta Catenin/analysis*

Desmoid type fibromatosis is a benign fibroblastic tumor arising from the fascia or musculoaponeurosis. It may occur in various locations, but most commonly in the shoulder girdle and neck; to our knowledge, there has been no reported case originating from a facet joint of the spine. We report CT and MR imaging findings of a desmoid type fibromatosis, involving the facet joint of the L3-4 spine with bone involvement.
Adult ; Biopsy ; Fibromatosis, Aggressive/*diagnosis ; Humans ; *Lumbar Vertebrae ; Magnetic Resonance Imaging ; Male ; Spinal Neoplasms/*diagnosis ; *Zygapophyseal Joint